부산대학교 도서관

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by the presence of proliferative lesions throughout the body. Management of TSC is challenging because patients have a multifaceted systemic illness with prominent neurological and developmental impact as well as potentially severe kidney, heart and lung phenotypes; however, every organ system can be involved. Adequate care for patients with TSC requires a coordinated effort involving a multidisciplinary team of clinicians and support staff. This clinical practice recommendation was developed by nephrologists, urologists, paediatric radiologists, interventional radiologists, geneticists, pathologists, and patient and family group representatives, with a focus on TSC-associated kidney manifestations. Careful monitoring of kidney function and assessment of kidney structural lesions by imaging enable early interventions that can preserve kidney function through targeted approaches. Here, we summarize the current evidence and present recommendations for the multidisciplinary management of kidney involvement in TSC. Care for patients with tuberous sclerosis complex (TSC) should be coordinated by a multidisciplinary team. This Consensus Statement, which involved input from nephrologists, urologists, geneticists, radiologists, interventional radiologists and pathologists as well as patient representatives, provides an overview of TSC-associated kidney manifestations and outlines current recommendations for the management of kidney involvement in TSC. [ABSTRACT FROM AUTHOR]